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A batch-scale electro-Fenton (EF) process was performed using graphite anode and waste battery-based Fe -Mn -Zn/C electrocatalyst coated on low-cost graphite felt cathode. The effectiveness of the EF's performance was evident with around 83.9 + 4.1% removal of 20 mg/L of sodium-dodecyl sulfate surfactant (SDS) at an optimum current density (CD) of 5.0 mA/cm2, Na2SO4 of 0.05 M, initial pH of 7.2, and electrolysis time of 180 min. Moreover, nearly 1.78-fold more removal of SDS was achieved in EF than in the electro-oxidation process operated without any catalyst. The operating cost of 0.35 $ of per m3 per order was needed to treat SDS wastewater. The remediation of SDS follows pseudo-first-order kinetics with a rate constant of 0.0095 min−1. Additionally, 90.3 + 2.1% of SDS and 57 + 2.6% of total organic carbon (TOC) removal was attained during 240 min of treatment time in secondary treated real wastewater; hence, additional 60 min of treatment time is required for effectively treating real wastewater than synthetic wastewater. Thus, EF is effective with battery waste-derived magnetic catalyst for treating wastewater containing SDS, which can lead to achieving sustainable environmental goals. HIGHLIGHTS The current work demonstrates a waste-to-treat-waste approach.; Magnetic Fe–Mn–Zn/C catalyst derived from battery waste was used in electro-Fenton.; Efficacious degradation of surfactant was obtained with a battery waste catalyst.; The kinetics of removal obtained a pseudo-first-order kinetic model.; Lauric acid was formed during sodium dodecyl sulfate degradation.;

Pompe disease is a progressive myopathy resulting from deficiency in lysosomal enzyme acid α-glucosidase (GAA), which leads to glycogen accumulation in lysosomes primarily in skeletal and cardiac muscle. Enzyme replacement therapy (ERT) with recombinant human (rh) GAA works well in alleviating the cardiomyopathy; however, many patients continue to have progressive muscle weakness. The purpose of this study was to evaluate the effectiveness of a respiratory training combined with 24-week supervised resistance training program on muscle strength (measured by Biodex)), and respiratory function including maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP) in subjects with late onset Pompe disease receiving ERT. Ten subjects participated in a 24-week resistance exercise program, three times per week, in addition to respiratory muscle exercise training six days per week. Overall, at the end of the resistance training program, as measured by Biodex dynamometry, the leg extensor strength improved by 10.5 ± 3.2Nm. (2O (p = 0.03) and the MEP by 6.4 ± 4.4 (p = 0.16). The exercise training significantly improved the trajectories of MIP and 6 MWT outcomes but not FVC when compared with the natural history data available in 6 individuals. These pilot results indicate that resistance training combined with respiratory training and ERT had a positive effect on muscular strength, functional capacity, and respiratory function in patients with late-onset Pompe disease and might be considered as a potential adjunct therapy in this population.

Abstract Wetlands are one of the most critical components of an ecosystem, supporting many ecological niches and a rich diversity of flora and fauna. The ecological significance of these sites makes it imperative to study the changes in their inundation extent and propose necessary measures for their conservation. This study analyzes all 64 Ramsar sites in China based on their inundation patterns using Landsat imagery from 1991 to 2020. Annual composites were generated using the short-wave infrared thresholding technique from June to September to create inundation maps. The analysis was carried out on each Ramsar site individually to account for its typical behavior due to regional geographical and climatic conditions. The results of the inundation analysis for each site were subjected to the Mann–Kendall test to determine their trends. The analysis showed that 8 sites exhibited a significantly decreasing trend, while 14 sites displayed a significantly increasing trend. The accuracy of the analysis ranged from a minimum of 72.0% for Hubei Wang Lake to a maximum of 98.0% for Zhangye Heihe Wetland National Nature Reserve. The average overall accuracy of the sites was found to be 90.0%. The findings emphasize the necessity for conservation strategies and policies for Ramsar sites.

Prader-Willi syndrome (PWS) is an imprinting genetic disorder characterized by lack of expression of genes on the paternal chromosome 15q11-q13 region. Growth hormone (GH) replacement positively influences stature and body composition in PWS. Our hypothesis was that early diagnosis delays onset of obesity in PWS. We studied 352 subjects with PWS, recruited from the NIH Rare Disease Clinical Research Network, to determine if age at diagnosis, ethnicity, gender, and PWS molecular class influenced the age they first become heavy, as determined by their primary care providers, and the age they first developed an increased appetite and began seeking food. The median ages that children with PWS became heavy were 10 years, 6 years and 4 years for age at diagnosis < 1 year, between 1 and 3 years, and greater than 3 years of age, respectively. The age of diagnosis and ethnicity were significant factors influencing when PWS children first became heavy (p < 0.01), however gender and the PWS molecular class had no influence. Early diagnosis delayed the onset of becoming heavy in individuals with PWS, permitting early GH and other treatment, thus reducing the risk of obesity-associated co-morbidities. Non-white individuals had an earlier onset of becoming heavy.

Prader-Willi syndrome (PWS) is an imprinting genetic disorder characterized by lack of expression of genes on the paternal chromosome 15q11-q13 region. Growth hormone (GH) replacement positively influences stature and body composition in PWS. Our hypothesis was that early diagnosis delays onset of obesity in PWS. We studied 352 subjects with PWS, recruited from the NIH Rare Disease Clinical Research Network, to determine if age at diagnosis, ethnicity, gender, and PWS molecular class influenced the age they first become heavy, as determined by their primary care providers, and the age they first developed an increased appetite and began seeking food. The median ages that children with PWS became heavy were 10 years, 6 years and 4 years for age at diagnosis < 1 year, between 1 and 3 years, and greater than 3 years of age, respectively. The age of diagnosis and ethnicity were significant factors influencing when PWS children first became heavy (p < 0.01), however gender and the PWS molecular class had no influence. Early diagnosis delayed the onset of becoming heavy in individuals with PWS, permitting early GH and other treatment, thus reducing the risk of obesity-associated co-morbidities. Non-white individuals had an earlier onset of becoming heavy.

Prader-Willi syndrome (PWS) is a genomic imprinting disorder characterized by infantile hypotonia with a poor suck and failure to thrive, hypogenitalism/hypogonadism, behavior and cognitive problems, hormone deficiencies, hyperphagia, and obesity. The Stanford Binet and Wechsler (WAIS-R; WISC-III) intelligence (IQ) tests were administered on 103 individuals with PWS from two separate cohorts [University of California, Irvine (UCI) (N = 56) and Vanderbilt University (N = 47)] and clinical information obtained including growth hormone (GH) treatment, PWS molecular classes, weight and height. Significantly higher IQ scores (p

A novel lyophilization (freeze-drying) technique has been developed to produce the petroleum hydrocarbon (PH)–degrading bacterial strain powders for the enhancement of bioremediation of PH-polluted soils. Four different PH-degrading strains (Paenarthrobacter ureafaciens, Klebsiella oxytoca, Pseudomonas citronellolis, and Enterobacter cloacae) isolated from a PH-polluted site were used for bacterial strain powder production. The modified lyophilization buffer contained skimmed milk powder, trehalose, sucrose, and glycerin. The water content, bacterial activity, bacterial survival factor, glass transition temperature, and surface characteristics were analyzed to determine the properties of the produced bacterial strain powders. Addition of 20% skimmed milk powder as an excipient and 15% trehalose as a cryoprotectant could significantly enhance the stability and survival factor of the bacterial strains. The incorporation of 0.75% sucrose and 0.05% glycerol into the basic formula of skimmed milk powder and trehalose, respectively, could allow the bacterial strain powder to be stored at ambient temperature due to the effective environmental isolation and continuous nutrient supplement capabilities of the two agents. Up to 95% of the bacterial survival could be obtained when 24 h of prefreezing at −80°C was applied as the first stage of the modified lyophilization process followed by the second stage of freeze-drying for 24 h. With this process, the depreciation rate was less than 1% after storing the powder for 6-month at 25°C. The glass transition temperature of the strain powder fell within the range of 10°C to 25°C, indicating that the optimized formulation for bacterial strain powder production could facilitate the strain storage at room temperature and produce diverse powder variants. Through the application of novel lyophilization process, more stable bacterial strain powder could be produced with a higher survival factor and a longer storage period. Up to 83% of total PH removal could be obtained in microcosms with the application of strain bacterial powders. [ABSTRACT FROM AUTHOR]

Spintronic devices that utilize spin transfer torque are promising for integrated memory applications. However, these devices face substantial energy consumption challenges due to the high current densities required for switching. Conversely, voltage‐driven spintronic devices, using capacitive displacement charge, can realize switching operations that are energy‐efficient (≈1–10 fJ bit−1). This work investigates switching based on voltage control of the interlayer exchange coupling in perpendicular magnetic anisotropy (PMA) multilayered heterostructures. Unlike previous works that utilized gating techniques that employ ionic transport mechanisms to control interlayer exchange coupling, this study employs electrostatic gating by using MgO, which is more compatible with modern spintronic‐based memories. These results suggest that the magnetization anisotropy, and the magnitude, and phase of the Ruderman‐Kittel‐Kasuya‐Yosida (RKKY) coupling function with spacer layer thickness can be controlled through electric gating, providing a promising avenue for the development of energy‐efficient magnetic data storage devices. [ABSTRACT FROM AUTHOR]

INTRODUCTION:Prader-Willi syndrome (PWS) is a complex genetic condition characterized by hyperphagia, hypotonia, low muscle mass, excess body fat, developmental delays, intellectual disability, behavioral problems, and growth hormone deficiency. This study evaluated the safety and efficacy of orally administered Diazoxide Choline Controlled-Release Tablets (DCCR) in subjects with PWS. METHOD:This was a single-center, Phase II study and included a 10-week Open-Label Treatment Period during which subjects were dose escalated, followed by a 4-week Double-Blind, Placebo-Controlled Treatment Period. RESULTS:Five female and eight male overweight or obese, adolescent and adult subjects with genetically-confirmed PWS with an average age of 15.5±2.9 years were enrolled in the study. There was a statistically significant reduction in hyperphagia at the end of the Open-Label Treatment Period (-4.32, n = 11, p = 0.006). The onset of effect on hyperphagia was rapid and greater reductions in hyperphagia were seen in subjects with moderate to severe Baseline hyperphagia (-5.50, n = 6, p = 0.03), in subjects treated with the highest dose (-6.25, n = 4, p = 0.08), and in subjects with moderate to severe Baseline hyperphagia treated with the highest dose (-7.83, n = 3, p = 0.09). DCCR treatment resulted in a reduction in the number of subjects displaying aggressive behaviors (-57.1%, n = 10, p = 0.01), clinically-relevant reductions in fat mass (-1.58 kg, n = 11, p = 0.02) and increases in lean body mass (2.26 kg, n = 11, p = 0.003). There was a corresponding decrease in waist circumference, and trends for improvements in lipids and insulin resistance. The most common adverse events were peripheral edema and transient increases in glucose. Many of the adverse events were common medical complications of PWS and diazoxide. CONCLUSION:DCCR treatment appears to address various unmet needs associated with PWS, including hyperphagia and aggressive behaviors in this proof-of-concept study. If the results were replicated in a larger scale study, DCCR may be a preferred therapeutic option for patients with PWS.

Inclusion body myopathy (IBM) associated with Paget disease of the bone and frontotemporal dementia or IBMPFD is an autosomal dominant degenerative disorder caused by mutations in the valosin-containing protein (VCP) gene. We aim to establish a detailed clinical phenotype of VCP disease amongst 35 (28 affected individuals, 7 presymptomatic gene carriers) individuals versus 14 unaffected first-degree relatives in 14 families to establish useful biomarkers for IBMPFD and identify the most meaningful tests for monitoring disease progression in future clinical trials. Comprehensive studies included the Inclusion Body Myositis Functional Rating Scale (IBMFRS) and fatigue severity scale questionairres, strength measurements using the Manual Muscle Test with Medical Research Council (MRC) scales, hand-held dynamometry using the microFET and Biodex dynamometers, 6 minute walk test (6MWT), and pulmonary function studies. Strong correlation was observed between the IBMFRS and measurements of muscle strength with dynamometry and the other functional tests, indicating that it may be utilized in long-term follow-up assessments due to its relative simplicity. This cross-section study represents the most comprehensive evaluation of individuals with VCP disease to date and provides a useful guide for evaluating and possible monitoring of muscle weakness and pulmonary function progression in this unique cohort of individuals.

Mutations in valosin-containing protein (VCP), an ATPase involved in protein degradation and autophagy, cause VCP disease, a progressive autosomal dominant adult onset multisystem proteinopathy. The goal of this study is to examine if phenotypic differences in this disorder could be explained by the specific gene mutations. We therefore studied 231 individuals (118 males and 113 females) from 36 families carrying 15 different VCP mutations. We analyzed the correlation between the different mutations and prevalence, age of onset and severity of myopathy, Paget's disease of bone (PDB), and frontotemporal dementia (FTD), and other comorbidities. Myopathy, PDB and FTD was present in 90%, 42% and 30% of the patients, respectively, beginning at an average age of 43, 41, and 56 years, respectively. Approximately 9% of patients with VCP mutations had an amyotrophic lateral sclerosis (ALS) phenotype, 4% had been diagnosed with Parkinson's disease (PD), and 2% had been diagnosed with Alzheimer's disease (AD). Large interfamilial and intrafamilial variation made establishing correlations difficult. We did not find a correlation between the mutation type and the incidence of any of the clinical features associated with VCP disease, except for the absence of PDB with the R159C mutation in our cohort and R159C having a later age of onset of myopathy compared with other molecular subtypes.